Yoshikawa H, Sakuragawa N, Nitta H
Division of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, Tokyo.
No To Hattatsu. 1990 Jan;22(1):9-15.
We reported clinical features and CT, MRI findings on cerebellum and brain stem in nine patients with congenital cerebellar ataxia (CCA) which was diagnosed by Hagberg's criteria. The patients were aged from 2 years to 14 years; five males and four females. CT and MRI were performed in nine cases and six cases, respectively. The patients were compared with sixty normal controls on CT, and with eight normal controls on MRI, aged from 1 year to 15 years. Five cases without perinatal brain insults showed abnormal findings in the cerebellum on CT and MRI. One case out of five cases showed abnormal finding only by MRI and not apparent by CT. Four cases with neonatal asphyxia did not show abnormal findings in the cerebellum. But the measurements of CT and MRI disclosed slightly abnormal values. Cerebrum and brain stem were though to be almost normal except for cavum Vergae of one case. There were no close correlation between clinical symptoms and abnormal findings in the cerebellum on CT and MRI. The etiology of CCA was thought to be heterogeneous, judging from CT and MRI. At least there were two causes; prenatal brain insults and perinatal brain insults. The degree of cerebellar lesion was thought to be various by its etiology.
我们报告了9例根据哈格伯格标准诊断为先天性小脑共济失调(CCA)患者的临床特征以及小脑和脑干的CT、MRI表现。患者年龄从2岁至14岁;男性5例,女性4例。分别对9例患者进行了CT检查,6例患者进行了MRI检查。将这些患者与60名年龄在1岁至15岁的正常对照者进行CT对比,与8名正常对照者进行MRI对比。5例无围产期脑损伤的患者在CT和MRI上显示小脑有异常表现。5例中有1例仅MRI显示异常,CT未显示明显异常。4例新生儿窒息患者小脑未显示异常表现。但CT和MRI测量显示数值略有异常。除1例有透明隔腔外,大脑和脑干被认为基本正常。CT和MRI上小脑的异常表现与临床症状之间无密切相关性。从CT和MRI判断,CCA的病因被认为是异质性的。至少有两个原因:产前脑损伤和围产期脑损伤。小脑病变的程度因病因不同而各异。